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Volumn 361, Issue 9358, 2003, Pages 681-689

Cystic fibrosis

Author keywords

[No Author keywords available]

Indexed keywords

ANTIBIOTIC AGENT; CHLORIDE CHANNEL;

EID: 0037460729     PISSN: 01406736     EISSN: None     Source Type: Journal    
DOI: 10.1016/S0140-6736(03)12567-6     Document Type: Conference Paper
Times cited : (935)

References (140)
  • 1
    • 0024424270 scopus 로고
    • Identification of the cystic fibrosis gene: Cloning and characterization of complementary DNA
    • Riordan J.R., Rommens J.M., Kerem B.S., et al. Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. Science. 245:1989;1066-1073.
    • (1989) Science , vol.245 , pp. 1066-1073
    • Riordan, J.R.1    Rommens, J.M.2    Kerem, B.S.3
  • 2
    • 0024453308 scopus 로고
    • Identification of the cystic fibrosis gene: Chromosome walking and jumping
    • Rommens J.M., Iannuzzi M.C., Kerem B.S., et al. Identification of the cystic fibrosis gene: chromosome walking and jumping. Science. 245:1989;1059-1065.
    • (1989) Science , vol.245 , pp. 1059-1065
    • Rommens, J.M.1    Iannuzzi, M.C.2    Kerem, B.S.3
  • 3
    • 0025760318 scopus 로고
    • Genomic DNA sequence of the cystic fibrosis transmembrane conductance regulator
    • Zielinski J., Rozmahel R., Bozon D., et al. Genomic DNA sequence of the cystic fibrosis transmembrane conductance regulator. Genomics. 10:1991;241-248.
    • (1991) Genomics , vol.10 , pp. 241-248
    • Zielinski, J.1    Rozmahel, R.2    Bozon, D.3
  • 4
    • 0026523829 scopus 로고
    • Cystic fibrosis: Molecular biology and therapeutic implications
    • Collins F.S. Cystic fibrosis: molecular biology and therapeutic implications. Science. 256:1992;774-779.
    • (1992) Science , vol.256 , pp. 774-779
    • Collins, F.S.1
  • 6
    • 0036120193 scopus 로고    scopus 로고
    • The cystic fibrosis transmembrane conductance regulator: An intriguing protein with pleiotropic functions
    • Vankeerberghen A., Cuppens H., Cassiman J.J. The cystic fibrosis transmembrane conductance regulator: an intriguing protein with pleiotropic functions. J Cystic Fibrosis. 1:2002;13-29.
    • (2002) J Cystic Fibrosis , vol.1 , pp. 13-29
    • Vankeerberghen, A.1    Cuppens, H.2    Cassiman, J.J.3
  • 7
    • 0027517995 scopus 로고
    • Correlation between genotype and phenotype in cystic fibrosis
    • Correlation between genotype and phenotype in cystic fibrosis. N Engl J Med. 329:1993;1308-1313.
    • (1993) N Engl J Med , vol.329 , pp. 1308-1313
  • 8
    • 0028229223 scopus 로고
    • The origin of the major cystic fibrosis mutation (delta F508) in European populations
    • Morral N., Bertranpetit J., Estivill X., et al. The origin of the major cystic fibrosis mutation (delta F508) in European populations. Nat Genet. 7:1994;169-175.
    • (1994) Nat Genet , vol.7 , pp. 169-175
    • Morral, N.1    Bertranpetit, J.2    Estivill, X.3
  • 9
    • 0028177981 scopus 로고
    • Delta F508 genotype does not predict disease severity in an ethnically diverse cystic fibrosis population
    • Lester L.A., Kraut J., Lloyd-Still J., et al. Delta F508 genotype does not predict disease severity in an ethnically diverse cystic fibrosis population. Pediatrics. 93:1994;114-118.
    • (1994) Pediatrics , vol.93 , pp. 114-118
    • Lester, L.A.1    Kraut, J.2    Lloyd-Still, J.3
  • 10
    • 0035213684 scopus 로고    scopus 로고
    • Chloride conductance and genetic background modulate the cystic fibrosis phenotype of Delta F508 homozygous twins and siblings
    • Bronsveld I., Mekus F., Bijman J., et al. Chloride conductance and genetic background modulate the cystic fibrosis phenotype of Delta F508 homozygous twins and siblings. J Clin Invest. 108:2001;1705-1715.
    • (2001) J Clin Invest , vol.108 , pp. 1705-1715
    • Bronsveld, I.1    Mekus, F.2    Bijman, J.3
  • 11
    • 0032935226 scopus 로고    scopus 로고
    • HLA class II polymorphism in cystic fibrosis: A possible modifier of pulmonary phenotype
    • Aron Y., Polla B.S., Bienvenu T., et al. HLA class II polymorphism in cystic fibrosis: a possible modifier of pulmonary phenotype. Am J Respir Crit Care Med. 159:1999;1464-1468.
    • (1999) Am J Respir Crit Care Med , vol.159 , pp. 1464-1468
    • Aron, Y.1    Polla, B.S.2    Bienvenu, T.3
  • 12
    • 0034523682 scopus 로고    scopus 로고
    • Airway NO levels in CF patients are related to a polymorphism in the neuronal NO synthase (NOS1) gene
    • Grasemann H., Knauer N., Eijmes J., et al. Airway NO levels in CF patients are related to a polymorphism in the neuronal NO synthase (NOS1) gene. Am J Respir Crit Care Med. 162:2000;2172-2176.
    • (2000) Am J Respir Crit Care Med , vol.162 , pp. 2172-2176
    • Grasemann, H.1    Knauer, N.2    Eijmes, J.3
  • 13
    • 0028473632 scopus 로고
    • Allotypes of alpha1-antitrypsin in cystic fibrosis patients homozygous and heterozygous for delta F508
    • Döring G., Krogh-Johansen H., Weidinger S., et al. Allotypes of alpha1-antitrypsin in cystic fibrosis patients homozygous and heterozygous for delta F508. Pediatr Pulmonol. 18:1994;3-7.
    • (1994) Pediatr Pulmonol , vol.18 , pp. 3-7
    • Döring, G.1    Krogh-Johansen, H.2    Weidinger, S.3
  • 14
    • 0035153395 scopus 로고    scopus 로고
    • Association of alpha(1)-antichymotrypsin deficiency with milder lung disease in patients with cystic fibrosis
    • Mahadeva R., Sharples L., Ross-Russell R.I., et al. Association of alpha(1)-antichymotrypsin deficiency with milder lung disease in patients with cystic fibrosis. Thorax. 56:2001;53-58.
    • (2001) Thorax , vol.56 , pp. 53-58
    • Mahadeva, R.1    Sharples, L.2    Ross-Russell, R.I.3
  • 15
    • 0032695668 scopus 로고    scopus 로고
    • Association of mannose-binding lectin gene heterogeneity with severity of lung disease and survival in cystic fibrosis
    • Garred P., Pressler T., Madsen H.O., et al. Association of mannose-binding lectin gene heterogeneity with severity of lung disease and survival in cystic fibrosis. J Clin Invest. 104:1999;431-437.
    • (1999) J Clin Invest , vol.104 , pp. 431-437
    • Garred, P.1    Pressler, T.2    Madsen, H.O.3
  • 16
    • 0036020992 scopus 로고    scopus 로고
    • 2-adrenoreceptor polymorphisms as modifiers of lung disease in cystic fibrosis
    • 2-adrenoreceptor polymorphisms as modifiers of lung disease in cystic fibrosis. Pharmacogenetics. 12:2002;347-353.
    • (2002) Pharmacogenetics , vol.12 , pp. 347-353
    • Büscher, R.1    Eilmes, K.J.2    Grasemann, H.3
  • 17
    • 0033028607 scopus 로고    scopus 로고
    • Detection of a cystic fibrosis modifier locus for meconium ileus on human chromosome 19q13
    • Zielenski J., Corey M., Rozmahel R., et al. Detection of a cystic fibrosis modifier locus for meconium ileus on human chromosome 19q13. Nat Genet. 22:1999;128-129.
    • (1999) Nat Genet , vol.22 , pp. 128-129
    • Zielenski, J.1    Corey, M.2    Rozmahel, R.3
  • 18
    • 0023789980 scopus 로고
    • Evidence for reduced Cl- and increased Na+ permeability in cystic fibrosis human primary cell cultures
    • Boucher R., Cotton C.U., Gatzy J.T., et al. Evidence for reduced Cl- and increased Na+ permeability in cystic fibrosis human primary cell cultures. J Physiol. 404:1988;77-103.
    • (1988) J Physiol , vol.404 , pp. 77-103
    • Boucher, R.1    Cotton, C.U.2    Gatzy, J.T.3
  • 19
    • 0032478144 scopus 로고    scopus 로고
    • Chloride channel and chloride conductance regulator domains of CFTR, the cystic fibrosis transmembrane conductance regulator
    • Schwiebert E.M., Morales M.M., Devidas S., et al. Chloride channel and chloride conductance regulator domains of CFTR, the cystic fibrosis transmembrane conductance regulator. Proc Natl Acad Sci USA. 95:1998;2674-2689.
    • (1998) Proc Natl Acad Sci USA , vol.95 , pp. 2674-2689
    • Schwiebert, E.M.1    Morales, M.M.2    Devidas, S.3
  • 20
    • 0033581885 scopus 로고    scopus 로고
    • Activation of the epithelial Na+ channel (ENaC) requires CFTR Cl- channel function
    • Reddy M.M., Light M.J., Quinton P.M. Activation of the epithelial Na+ channel (ENaC) requires CFTR Cl- channel function. Nature. 402:1999;301-304.
    • (1999) Nature , vol.402 , pp. 301-304
    • Reddy, M.M.1    Light, M.J.2    Quinton, P.M.3
  • 21
    • 0025874599 scopus 로고
    • Activation by extracellular nucleotides of chloride secretion in the airway epithelia of patients with cystic fibrosis
    • Knowles M.R., Clarke L.L., Boucher R.C. Activation by extracellular nucleotides of chloride secretion in the airway epithelia of patients with cystic fibrosis. N Engl J Med. 325:1991;533-538.
    • (1991) N Engl J Med , vol.325 , pp. 533-538
    • Knowles, M.R.1    Clarke, L.L.2    Boucher, R.C.3
  • 24
    • 0028914085 scopus 로고
    • Early pulmonary inflammation in infants with cystic fibrosis
    • Khan T.Z., Wagener J.S., Bost T., et al. Early pulmonary inflammation in infants with cystic fibrosis. Am J Respir Crit Care Med. 151:1995;1075-1082.
    • (1995) Am J Respir Crit Care Med , vol.151 , pp. 1075-1082
    • Khan, T.Z.1    Wagener, J.S.2    Bost, T.3
  • 25
    • 0030909647 scopus 로고    scopus 로고
    • Early alterations in airway mucociliary clearance and inflammation of the lamina propria in CF mice
    • Zahm J.M., Gaillard D., Dupuit F., et al. Early alterations in airway mucociliary clearance and inflammation of the lamina propria in CF mice. Am J Physiol. 272:1997;C853-C859.
    • (1997) Am J Physiol , vol.272
    • Zahm, J.M.1    Gaillard, D.2    Dupuit, F.3
  • 26
    • 0033844485 scopus 로고    scopus 로고
    • Inflammation and infection in naive human cystic fibrosis airway grafts
    • Tirouvanziam R., de Bentzmann S., Hubeau C., et al. Inflammation and infection in naive human cystic fibrosis airway grafts. Am J Respir Cell Mol Biol. 23:2000;121-127.
    • (2000) Am J Respir Cell Mol Biol , vol.23 , pp. 121-127
    • Tirouvanziam, R.1    De Bentzmann, S.2    Hubeau, C.3
  • 27
    • 0030768159 scopus 로고    scopus 로고
    • Lower airway inflammation in infants and young children with cystic fibrosis
    • Armstrong D.S., Grimwood K., Carlin J.B., et al. Lower airway inflammation in infants and young children with cystic fibrosis. Am J Respir Crit Care Med. 156:1997;1197-1204.
    • (1997) Am J Respir Crit Care Med , vol.156 , pp. 1197-1204
    • Armstrong, D.S.1    Grimwood, K.2    Carlin, J.B.3
  • 28
    • 0029372481 scopus 로고
    • Normal bronchial epithelial cells constitutively produce the anti-inflammatory cytokine interleukin-10, which is downregulated in cystic fibrosis
    • Bonfield T.L., Konstan M.W., Burfeind P., et al. Normal bronchial epithelial cells constitutively produce the anti-inflammatory cytokine interleukin-10, which is downregulated in cystic fibrosis. Am J Respir Cell Mol Biol. 13:1995;257-261.
    • (1995) Am J Respir Cell Mol Biol , vol.13 , pp. 257-261
    • Bonfield, T.L.1    Konstan, M.W.2    Burfeind, P.3
  • 29
    • 0033456217 scopus 로고    scopus 로고
    • IL-10 attenuates excessive inflammation in chronic Pseudomonas infection in mice
    • Chmiel J.F., Konstan M.W., Knesebeck J.E., et al. IL-10 attenuates excessive inflammation in chronic Pseudomonas infection in mice. Am J Respir Crit Care Med. 160:1999;2040-2047.
    • (1999) Am J Respir Crit Care Med , vol.160 , pp. 2040-2047
    • Chmiel, J.F.1    Konstan, M.W.2    Knesebeck, J.E.3
  • 30
    • 0035923599 scopus 로고    scopus 로고
    • Molecular basis for defective glycosylation and Pseudomonas pathogenesis in cystic fibrosis lung
    • Poschet J.F., Boucher J.C., Tatterson L., et al. Molecular basis for defective glycosylation and Pseudomonas pathogenesis in cystic fibrosis lung. Proc Natl Acad Sci USA. 98:2001;13972-13977.
    • (2001) Proc Natl Acad Sci USA , vol.98 , pp. 13972-13977
    • Poschet, J.F.1    Boucher, J.C.2    Tatterson, L.3
  • 31
    • 0028920643 scopus 로고
    • Cystic fibrosis epithelial cells have a receptor for pathogenic bacteria on their apical surface
    • Imundo L., Barasch J., Prince A., et al. Cystic fibrosis epithelial cells have a receptor for pathogenic bacteria on their apical surface. Proc Natl Acad Sci USA. 92:1995;3019-3023.
    • (1995) Proc Natl Acad Sci USA , vol.92 , pp. 3019-3023
    • Imundo, L.1    Barasch, J.2    Prince, A.3
  • 32
    • 0030021470 scopus 로고    scopus 로고
    • Role of mutant CFTR in hypersusceptibility of cystic fibrosis patients to lung infections
    • Pier G.B., Grout M., Zaidi T.S., et al. Role of mutant CFTR in hypersusceptibility of cystic fibrosis patients to lung infections. Science. 271:1996;64-67.
    • (1996) Science , vol.271 , pp. 64-67
    • Pier, G.B.1    Grout, M.2    Zaidi, T.S.3
  • 33
    • 0029870085 scopus 로고    scopus 로고
    • Cystic fibrosis airway epithelia fail to kill bacteria because of abnormal airway surface fluid
    • Smith J.J., Travis S.M., Greenberg E.P., et al. Cystic fibrosis airway epithelia fail to kill bacteria because of abnormal airway surface fluid. Cell. 85:1996;229-236.
    • (1996) Cell , vol.85 , pp. 229-236
    • Smith, J.J.1    Travis, S.M.2    Greenberg, E.P.3
  • 34
    • 0035178729 scopus 로고    scopus 로고
    • Altered NaCl concentration of airway surface liquid in cystic fibrosis
    • Widdicombe J.H. Altered NaCl concentration of airway surface liquid in cystic fibrosis. Pflügers Arch Eur J Physiol. 443:(suppl 1):2001;S8-S10.
    • (2001) Pflügers Arch Eur J Physiol , vol.443 , Issue.SUPPL. 1
    • Widdicombe, J.H.1
  • 35
    • 0033560645 scopus 로고    scopus 로고
    • Transfer of a cathelicidin peptide antibiotic gene restores bacterial killing in a cystic fibrosis xenograft model
    • Bals R., Weiner D.J., Meegalla R.L., et al. Transfer of a cathelicidin peptide antibiotic gene restores bacterial killing in a cystic fibrosis xenograft model. J Clin Invest. 103:1999;1113-1117.
    • (1999) J Clin Invest , vol.103 , pp. 1113-1117
    • Bals, R.1    Weiner, D.J.2    Meegalla, R.L.3
  • 36
    • 0035132659 scopus 로고    scopus 로고
    • Noninvasive in vivo fluorescence measurement of airway-surface liquid depth, salt concentration, and pH
    • Jayaraman S., Song Y., Vetrivel L., et al. Noninvasive in vivo fluorescence measurement of airway-surface liquid depth, salt concentration, and pH. J Clin Invest. 107:2001;317-324.
    • (2001) J Clin Invest , vol.107 , pp. 317-324
    • Jayaraman, S.1    Song, Y.2    Vetrivel, L.3
  • 37
    • 0032433707 scopus 로고    scopus 로고
    • Evidence for periciliary liquid layer depletion, not abnormal ion composition, in the pathogenesis of cystic fibrosis airways disease
    • Matsui H., Grubb B.R., Tarran R., et al. Evidence for periciliary liquid layer depletion, not abnormal ion composition, in the pathogenesis of cystic fibrosis airways disease. Cell. 95:1998;1005-1015.
    • (1998) Cell , vol.95 , pp. 1005-1015
    • Matsui, H.1    Grubb, B.R.2    Tarran, R.3
  • 38
    • 0036167851 scopus 로고    scopus 로고
    • Effects of reduced mucus oxygen concentration in airway Pseudomonas infections of cystic fibrosis patients
    • Worlitzsch D., Tarran R., Ulrich M., et al. Effects of reduced mucus oxygen concentration in airway Pseudomonas infections of cystic fibrosis patients. J Clin Invest. 109:2002;317-325.
    • (2002) J Clin Invest , vol.109 , pp. 317-325
    • Worlitzsch, D.1    Tarran, R.2    Ulrich, M.3
  • 39
    • 0033056054 scopus 로고    scopus 로고
    • Mucoid conversion of Pseudomonas aeruginosa by hydrogen peroxide: A mechanism for virulence activation in the cystic fibrosis lung
    • Mathee K., Ciofu O., Sternberg C., et al. Mucoid conversion of Pseudomonas aeruginosa by hydrogen peroxide: a mechanism for virulence activation in the cystic fibrosis lung. Microbiology. 145:1999;1349-1357.
    • (1999) Microbiology , vol.145 , pp. 1349-1357
    • Mathee, K.1    Ciofu, O.2    Sternberg, C.3
  • 40
    • 0028015883 scopus 로고
    • Bronchoalveolar lavage findings in cystic fibrosis patients with stable, clinically mild lung disease suggest ongoing infection and inflammation
    • Konstan M.W., Hilliard K.A., Norvell T.M., et al. Bronchoalveolar lavage findings in cystic fibrosis patients with stable, clinically mild lung disease suggest ongoing infection and inflammation. Am J Respir Crit Care Med. 150:1994;448-454.
    • (1994) Am J Respir Crit Care Med , vol.150 , pp. 448-454
    • Konstan, M.W.1    Hilliard, K.A.2    Norvell, T.M.3
  • 41
    • 0021917723 scopus 로고
    • Pancreatic fluid secretion and protein hyperconcentration in cystic fibrosis
    • Kopelman H., Durie P., Gaskin K., et al. Pancreatic fluid secretion and protein hyperconcentration in cystic fibrosis. N Engl J Med. 312:1985;329-334.
    • (1985) N Engl J Med , vol.312 , pp. 329-334
    • Kopelman, H.1    Durie, P.2    Gaskin, K.3
  • 42
    • 0025720681 scopus 로고
    • The cystic fibrosis gene and resting energy expenditure
    • Fried M.D., Durie P.R., Tsui L.C., et al. The cystic fibrosis gene and resting energy expenditure. J Pediatr. 119:1991;913-916.
    • (1991) J Pediatr , vol.119 , pp. 913-916
    • Fried, M.D.1    Durie, P.R.2    Tsui, L.C.3
  • 43
    • 0028314401 scopus 로고
    • Diabetes mellitus in Danish cystic fibrosis patients: Prevalence and late diabetic complications
    • Lanng S., Thornsteinsson B., Lund-Anderen C., et al. Diabetes mellitus in Danish cystic fibrosis patients: prevalence and late diabetic complications. Acta Paediatr. 83:1994;72-77.
    • (1994) Acta Paediatr , vol.83 , pp. 72-77
    • Lanng, S.1    Thornsteinsson, B.2    Lund-Anderen, C.3
  • 44
    • 0030760747 scopus 로고    scopus 로고
    • Insulin resistance with altered secretory kinetics and reduced proinsulin in cystic fibrosis patients
    • Holl R.W., Wolf A., Thon A., et al. Insulin resistance with altered secretory kinetics and reduced proinsulin in cystic fibrosis patients. J Pediatr Gastroenterol Nutr. 25:1997;188-193.
    • (1997) J Pediatr Gastroenterol Nutr , vol.25 , pp. 188-193
    • Holl, R.W.1    Wolf, A.2    Thon, A.3
  • 45
    • 0029097769 scopus 로고
    • Male fertility in cystic fibrosis
    • Dodge J.A. Male fertility in cystic fibrosis. Lancet. 346:1995;587-588.
    • (1995) Lancet , vol.346 , pp. 587-588
    • Dodge, J.A.1
  • 46
    • 0015885904 scopus 로고
    • Water and electrolytes in cervical mucus from patients with cystic fibrosis
    • Kopito L.E., Kosasky H.J., Shwachman H. Water and electrolytes in cervical mucus from patients with cystic fibrosis. Fertil Steril. 24:1973;512-516.
    • (1973) Fertil Steril , vol.24 , pp. 512-516
    • Kopito, L.E.1    Kosasky, H.J.2    Shwachman, H.3
  • 47
    • 0031900652 scopus 로고    scopus 로고
    • The diagnosis of cystic fibrosis: A consensus statement
    • Rosenstein B.J., Cutting G.R. The diagnosis of cystic fibrosis: a consensus statement. J Pediatr. 132:1998;589-595.
    • (1998) J Pediatr , vol.132 , pp. 589-595
    • Rosenstein, B.J.1    Cutting, G.R.2
  • 48
    • 0031016388 scopus 로고    scopus 로고
    • Current concepts: The diagnosis of cystic fibrosis
    • Stern R.C. Current concepts: the diagnosis of cystic fibrosis. N Engl J Med. 336:1997;487-491.
    • (1997) N Engl J Med , vol.336 , pp. 487-491
    • Stern, R.C.1
  • 49
    • 0031900650 scopus 로고    scopus 로고
    • Making the diagnosis of cystic fibrosis
    • Wilmott R.W. Making the diagnosis of cystic fibrosis. J Pediatr. 132:1998;563-565.
    • (1998) J Pediatr , vol.132 , pp. 563-565
    • Wilmott, R.W.1
  • 50
    • 0025772974 scopus 로고
    • Ion transport abnormalities in rectal suction biopsies from children with cystic fibrosis
    • Veeze H.J., Sinaasappel M., Bijman J., et al. Ion transport abnormalities in rectal suction biopsies from children with cystic fibrosis. Gastroenterology. 101:1991;398-3403.
    • (1991) Gastroenterology , vol.101 , pp. 398-3403
    • Veeze, H.J.1    Sinaasappel, M.2    Bijman, J.3
  • 51
    • 0033866283 scopus 로고    scopus 로고
    • Time to think again: Cystic fibrosis is not an "all or none" disease
    • Bush A., Wallis C. Time to think again: cystic fibrosis is not an "all or none" disease. Pediatr Pulmonol. 30:2000;139-144.
    • (2000) Pediatr Pulmonol , vol.30 , pp. 139-144
    • Bush, A.1    Wallis, C.2
  • 52
    • 0013983024 scopus 로고
    • Stool trypsin and chymotrypsin: Value in the diagnosis of pancreatic insufficiency in cystic fibrosis
    • Barbero G.J., Sibinga M.S., Marino J.M. Stool trypsin and chymotrypsin: value in the diagnosis of pancreatic insufficiency in cystic fibrosis. Am J Dis Child. 112:1966;536-540.
    • (1966) Am J Dis Child , vol.112 , pp. 536-540
    • Barbero, G.J.1    Sibinga, M.S.2    Marino, J.M.3
  • 53
    • 0032737592 scopus 로고    scopus 로고
    • Faecal elastase 1: A marker of exocrine pancreatic insufficiency in cystic fibrosis
    • Phillips I.J., Rowe D.J., Dewar P., et al. Faecal elastase 1: a marker of exocrine pancreatic insufficiency in cystic fibrosis. Ann Clin Biochem. 36:1999;739-742.
    • (1999) Ann Clin Biochem , vol.36 , pp. 739-742
    • Phillips, I.J.1    Rowe, D.J.2    Dewar, P.3
  • 54
    • 0000518360 scopus 로고
    • Paranasal sinuses in cystic fibrosis: Incidence of roentgen abnormalities
    • Gharib R., Allen R.P., Joos H.A., et al. Paranasal sinuses in cystic fibrosis: incidence of roentgen abnormalities. Am J Dis Child. 108:1964;499-502.
    • (1964) Am J Dis Child , vol.108 , pp. 499-502
    • Gharib, R.1    Allen, R.P.2    Joos, H.A.3
  • 55
    • 1842339924 scopus 로고    scopus 로고
    • Distinct spectrum of CFTR mutations in congenital absence of vas deferens
    • Dörk T., Dworniczak B., Aulehla-Scholz C., et al. Distinct spectrum of CFTR mutations in congenital absence of vas deferens. Hum Genet. 100:1997;365-377.
    • (1997) Hum Genet , vol.100 , pp. 365-377
    • Dörk, T.1    Dworniczak, B.2    Aulehla-Scholz, C.3
  • 57
    • 0034596493 scopus 로고    scopus 로고
    • Neonatal screening for cystic fibrosis in Brittany, France: Assessment of 10 years' experience and impact on prenatal diagnosis
    • Scotet V., de Braekeleer M., Roussey M., et al. Neonatal screening for cystic fibrosis in Brittany, France: assessment of 10 years' experience and impact on prenatal diagnosis. Lancet. 356:2000;789-794.
    • (2000) Lancet , vol.356 , pp. 789-794
    • Scotet, V.1    De Braekeleer, M.2    Roussey, M.3
  • 58
    • 0030803131 scopus 로고    scopus 로고
    • Nutritional benefits of neonatal screening for cystic fibrosis
    • Farrell P.M., Kosorok M.R., Laxova A., et al. Nutritional benefits of neonatal screening for cystic fibrosis. N Engl J Med. 337:1997;963-969.
    • (1997) N Engl J Med , vol.337 , pp. 963-969
    • Farrell, P.M.1    Kosorok, M.R.2    Laxova, A.3
  • 59
    • 17744372496 scopus 로고    scopus 로고
    • Early diagnosis of cystic fibrosis through neonatal screening prevents severe malnutrition and improves long-term growth
    • Farrell P.M., Kosorok M.R., Rock M.J., et al. Early diagnosis of cystic fibrosis through neonatal screening prevents severe malnutrition and improves long-term growth. Pediatrics. 107:2001;1-13.
    • (2001) Pediatrics , vol.107 , pp. 1-13
    • Farrell, P.M.1    Kosorok, M.R.2    Rock, M.J.3
  • 60
    • 0029833357 scopus 로고    scopus 로고
    • Nutrition and survival in cystic fibrosis
    • Elborn J.S., Bell S.C. Nutrition and survival in cystic fibrosis. Thorax. 51:1996;971-972.
    • (1996) Thorax , vol.51 , pp. 971-972
    • Elborn, J.S.1    Bell, S.C.2
  • 61
    • 0031267463 scopus 로고    scopus 로고
    • Acquisition of Pseudomonas aeruginosa in children with cystic fibrosis
    • Farrell P.M., Shen G., Splaingard M., et al. Acquisition of Pseudomonas aeruginosa in children with cystic fibrosis. Pediatrics. 100:1997;E2.
    • (1997) Pediatrics , vol.100 , pp. 2
    • Farrell, P.M.1    Shen, G.2    Splaingard, M.3
  • 62
    • 0034834535 scopus 로고    scopus 로고
    • Gene therapy in cystic fibrosis
    • Flotte T.R., Laube B.L. Gene therapy in cystic fibrosis. Chest. 120:2001;124S-131S.
    • (2001) Chest , vol.120
    • Flotte, T.R.1    Laube, B.L.2
  • 63
    • 0029100239 scopus 로고
    • A controlled study of adenoviral-vector-mediated gene transfer in the nasal epithelium of patients with cystic fibrosis
    • Knowles M.R., Hohneker K.W., Zhou Z., et al. A controlled study of adenoviral-vector-mediated gene transfer in the nasal epithelium of patients with cystic fibrosis. N Engl J Med. 333:1995;823-831.
    • (1995) N Engl J Med , vol.333 , pp. 823-831
    • Knowles, M.R.1    Hohneker, K.W.2    Zhou, Z.3
  • 64
    • 0033586375 scopus 로고    scopus 로고
    • Cationic lipid-mediated CFTR gene transfer to the lungs and nose of patients with cystic fibrosis: A double-blind placebo-controlled trial
    • Alton E.W., Stern M., Farley R., et al. Cationic lipid-mediated CFTR gene transfer to the lungs and nose of patients with cystic fibrosis: a double-blind placebo-controlled trial. Lancet. 353:1999;947-954.
    • (1999) Lancet , vol.353 , pp. 947-954
    • Alton, E.W.1    Stern, M.2    Farley, R.3
  • 65
    • 13344286312 scopus 로고    scopus 로고
    • Repeat administration of an adenovirus vector encoding cystic fibrosis transmembrane conductance regulator to the nasal epithelium of patients with cystic fibrosis
    • Zabner J., Ramsey B.W., Meeker D.P., et al. Repeat administration of an adenovirus vector encoding cystic fibrosis transmembrane conductance regulator to the nasal epithelium of patients with cystic fibrosis. J Clin Invest. 97:1996;1504-1511.
    • (1996) J Clin Invest , vol.97 , pp. 1504-1511
    • Zabner, J.1    Ramsey, B.W.2    Meeker, D.P.3
  • 66
    • 0035894583 scopus 로고    scopus 로고
    • Gentamicin in pharmocogenetic approach to treatment of cystic fibrosis
    • Hamilton J.W. Gentamicin in pharmocogenetic approach to treatment of cystic fibrosis. Lancet. 358:2001;2014-2016.
    • (2001) Lancet , vol.358 , pp. 2014-2016
    • Hamilton, J.W.1
  • 67
    • 0025242929 scopus 로고
    • Defective intracellular traffic and processing of CFTR is the molecular basis of most cystic fibrosis
    • Cheng S.H., Gregory R.J., Marshall J., et al. Defective intracellular traffic and processing of CFTR is the molecular basis of most cystic fibrosis. Cell. 63:1990;827-834.
    • (1990) Cell , vol.63 , pp. 827-834
    • Cheng, S.H.1    Gregory, R.J.2    Marshall, J.3
  • 68
    • 0031915434 scopus 로고    scopus 로고
    • Limited proteolysis as a probe for arrested conformational maturation of delta F508 CFTR
    • Zhang F., Kartner N., Lukacs G.L. Limited proteolysis as a probe for arrested conformational maturation of delta F508 CFTR. Nat Struct Biol. 5:1998;180-183.
    • (1998) Nat Struct Biol , vol.5 , pp. 180-183
    • Zhang, F.1    Kartner, N.2    Lukacs, G.L.3
  • 69
    • 0032588980 scopus 로고    scopus 로고
    • DeltaF508 CFTR protein expression in tissues from patients with cystic fibrosis
    • Kalin N., Claass A., Sommer M., et al. DeltaF508 CFTR protein expression in tissues from patients with cystic fibrosis. J Clin Invest. 103:1999;1379-1389.
    • (1999) J Clin Invest , vol.103 , pp. 1379-1389
    • Kalin, N.1    Claass, A.2    Sommer, M.3
  • 70
    • 0033918281 scopus 로고    scopus 로고
    • Cystic fibrosis F508del patients have apically localized CFTR in a reduced number of airway cells
    • Penque D., Mendes F., Beck S., et al. Cystic fibrosis F508del patients have apically localized CFTR in a reduced number of airway cells. Lab Invest. 80:2000;857-868.
    • (2000) Lab Invest , vol.80 , pp. 857-868
    • Penque, D.1    Mendes, F.2    Beck, S.3
  • 71
    • 0033625415 scopus 로고    scopus 로고
    • Activation of deltaF508 CFTR in a cystic fibrosis respiratory epithelial cell line by 4-phenylbutyrate, genistein and CPX
    • Andersson C., Roomans G.M. Activation of deltaF508 CFTR in a cystic fibrosis respiratory epithelial cell line by 4-phenylbutyrate, genistein and CPX. Eur Respir J. 15:2000;937-941.
    • (2000) Eur Respir J , vol.15 , pp. 937-941
    • Andersson, C.1    Roomans, G.M.2
  • 72
    • 0031889082 scopus 로고    scopus 로고
    • A pilot clinical trial of oral sodium 4-phenylbutyrate (Buphenyl) in deltaF508-homozygous cystic fibrosis patients: Partial restoration of nasal epithelial CFTR function
    • Rubenstein R.C., Zeitlin P.L. A pilot clinical trial of oral sodium 4-phenylbutyrate (Buphenyl) in deltaF508-homozygous cystic fibrosis patients: partial restoration of nasal epithelial CFTR function. Am J Respir Crit Care Med. 157:1998;484-490.
    • (1998) Am J Respir Crit Care Med , vol.157 , pp. 484-490
    • Rubenstein, R.C.1    Zeitlin, P.L.2
  • 73
    • 0036161237 scopus 로고    scopus 로고
    • A phase I randomized, multicenter trial of CPX in adult subjects with mild cystic fibrosis
    • McCarty N.A., Standaert T.A., Teresi M., et al. A phase I randomized, multicenter trial of CPX in adult subjects with mild cystic fibrosis. Pediatr Pulmonol. 33:2002;90-98.
    • (2002) Pediatr Pulmonol , vol.33 , pp. 90-98
    • McCarty, N.A.1    Standaert, T.A.2    Teresi, M.3
  • 74
    • 0035200620 scopus 로고    scopus 로고
    • Cell-based assay for high-throughput quantitative screening of CFTR chloride transport agonists
    • Galietta L.V., Jayaraman S., Verkman A.S. Cell-based assay for high-throughput quantitative screening of CFTR chloride transport agonists. Am J Physiol Cell Physiol. 281:2001;C1734-C1742.
    • (2001) Am J Physiol Cell Physiol , vol.281
    • Galietta, L.V.1    Jayaraman, S.2    Verkman, A.S.3
  • 75
    • 0034192547 scopus 로고    scopus 로고
    • Deletion of phenylalanine 508 causes attenuated phosphorylation-dependent activation of CFTR chloride channels
    • Wang F., Zeltwanger S., Hu S., et al. Deletion of phenylalanine 508 causes attenuated phosphorylation-dependent activation of CFTR chloride channels. J Physiol. 524:2000;637-648.
    • (2000) J Physiol , vol.524 , pp. 637-648
    • Wang, F.1    Zeltwanger, S.2    Hu, S.3
  • 76
    • 0025236867 scopus 로고
    • A pilot study of aerosolized amiloride for the treatment of lung disease in cystic fibrosis
    • Knowles M.R., Church N.L., Waltner W.E., et al. A pilot study of aerosolized amiloride for the treatment of lung disease in cystic fibrosis. N Engl J Med. 322:1990;1189-1194.
    • (1990) N Engl J Med , vol.322 , pp. 1189-1194
    • Knowles, M.R.1    Church, N.L.2    Waltner, W.E.3
  • 77
    • 0025874599 scopus 로고
    • Activation by extracellular nucleotides of chloride secretion in the airway epithelia of patients with cystic fibrosis
    • Knowles M.R., Clarke L.L., Boucher R.C. Activation by extracellular nucleotides of chloride secretion in the airway epithelia of patients with cystic fibrosis. N Engl J Med. 325:1991;533-538.
    • (1991) N Engl J Med , vol.325 , pp. 533-538
    • Knowles, M.R.1    Clarke, L.L.2    Boucher, R.C.3
  • 78
    • 0032833866 scopus 로고    scopus 로고
    • The effect of topical benzamil and amiloride on nasal potential difference in cystic fibrosis
    • Rodgers H.C., Knox A.J. The effect of topical benzamil and amiloride on nasal potential difference in cystic fibrosis. Eur Respir J. 14:1999;693-696.
    • (1999) Eur Respir J , vol.14 , pp. 693-696
    • Rodgers, H.C.1    Knox, A.J.2
  • 79
    • 0034896551 scopus 로고    scopus 로고
    • Safety of aerosolized INS 365 in patients with mild to moderate cystic fibrosis: Results of a phase I multi-center study
    • Noone P.G., Hamblett N., Accurso F., et al. Safety of aerosolized INS 365 in patients with mild to moderate cystic fibrosis: results of a phase I multi-center study. Pediatr Pulmonol. 32:2001;122-128.
    • (2001) Pediatr Pulmonol , vol.32 , pp. 122-128
    • Noone, P.G.1    Hamblett, N.2    Accurso, F.3
  • 80
    • 0013359613 scopus 로고    scopus 로고
    • Management of infection in cystic fibrosis
    • Döring G. Management of infection in cystic fibrosis. Hosp Pharm. 5:2002;65-69.
    • (2002) Hosp Pharm , vol.5 , pp. 65-69
    • Döring, G.1
  • 81
    • 0025077322 scopus 로고
    • Pseudomonas aeruginosa infection in cystic fibrosis and its management
    • Høiby N., Koch C. Pseudomonas aeruginosa infection in cystic fibrosis and its management. Thorax. 45:1990;881-884.
    • (1990) Thorax , vol.45 , pp. 881-884
    • Høiby, N.1    Koch, C.2
  • 82
    • 0027395872 scopus 로고
    • The changing epidemiology of cystic fibrosis
    • Fitzsimmons F.C. The changing epidemiology of cystic fibrosis. J Pediatr. 122:1993;1-9.
    • (1993) J Pediatr , vol.122 , pp. 1-9
    • Fitzsimmons, F.C.1
  • 83
    • 0019961926 scopus 로고
    • Antibiotic treatment of Staphylococcus aureus infection in cystic fibrosis
    • Szaff M., Hoiby N. Antibiotic treatment of Staphylococcus aureus infection in cystic fibrosis. Acta Paediatr Scand. 71:1982;21-26.
    • (1982) Acta Paediatr Scand , vol.71 , pp. 21-26
    • Szaff, M.1    Hoiby, N.2
  • 84
    • 0031958323 scopus 로고    scopus 로고
    • Persistent infection with small colony variant strains of Staphylococcus aureus in patients with cystic fibrosis
    • Kahl B., Herrmann M., Everding A.S., et al. Persistent infection with small colony variant strains of Staphylococcus aureus in patients with cystic fibrosis. J Infect Dis. 177:1998;1023-1029.
    • (1998) J Infect Dis , vol.177 , pp. 1023-1029
    • Kahl, B.1    Herrmann, M.2    Everding, A.S.3
  • 85
    • 0028350689 scopus 로고
    • Prognosis in cystic fibrosis treated with continuous flucloxacillin from the neonatal period
    • Weaver L.T., Green M.R., Nicholson K., et al. Prognosis in cystic fibrosis treated with continuous flucloxacillin from the neonatal period. Arch Dis Child. 70:1994;84-89.
    • (1994) Arch Dis Child , vol.70 , pp. 84-89
    • Weaver, L.T.1    Green, M.R.2    Nicholson, K.3
  • 86
    • 0035125425 scopus 로고    scopus 로고
    • Effect of continuous anti-staphylococcal therapy on the rate of P aeruginosa acquisition in patients with cystic fibrosis
    • Ratjen F., Comes G., Paul K., et al. Effect of continuous anti-staphylococcal therapy on the rate of P aeruginosa acquisition in patients with cystic fibrosis. Pediatr Pulmonol. 31:2001;13-16.
    • (2001) Pediatr Pulmonol , vol.31 , pp. 13-16
    • Ratjen, F.1    Comes, G.2    Paul, K.3
  • 87
    • 0036220978 scopus 로고    scopus 로고
    • Antibiotic prophylaxis in infants and young children with cystic fibrosis: A randomized controlled trial
    • Stutman H.R., Lieberman J.M., Nussbaum E., et al. Antibiotic prophylaxis in infants and young children with cystic fibrosis: a randomized controlled trial. J Pediatr. 140:2002;299-305.
    • (2002) J Pediatr , vol.140 , pp. 299-305
    • Stutman, H.R.1    Lieberman, J.M.2    Nussbaum, E.3
  • 88
    • 84921429624 scopus 로고    scopus 로고
    • Prophylactic antibiotics for cystic fibrosis (Cochrane Review)
    • Smyth A., Walters S. Prophylactic antibiotics for cystic fibrosis (Cochrane Review). Cochrane Database Syst Rev. 2:2000;CD001021.
    • (2000) Cochrane Database Syst Rev , vol.2 , pp. 001021
    • Smyth, A.1    Walters, S.2
  • 89
    • 0034685940 scopus 로고    scopus 로고
    • High frequency of hypermutable Pseudomonas aeruginosa in cystic fibrosis lung infection
    • Oliver A., Canton R., Campo P., et al. High frequency of hypermutable Pseudomonas aeruginosa in cystic fibrosis lung infection. Science. 288:2000;1251-1254.
    • (2000) Science , vol.288 , pp. 1251-1254
    • Oliver, A.1    Canton, R.2    Campo, P.3
  • 90
    • 0033773012 scopus 로고    scopus 로고
    • Antibiotic therapy against Pseudomonas aeruginosa in cystic fibrosis: A European consensus
    • Döring G., Conway S.P., Heijerman H.G., et al. Antibiotic therapy against Pseudomonas aeruginosa in cystic fibrosis: a European consensus. Eur Respir J. 16:2000;749-767.
    • (2000) Eur Respir J , vol.16 , pp. 749-767
    • Döring, G.1    Conway, S.P.2    Heijerman, H.G.3
  • 91
    • 0029781290 scopus 로고    scopus 로고
    • Adaptive resistance to tobramycin in Pseudomonas aeruginosa lung infection in cystic fibrosis
    • Barclay M.L., Begg E.J., Chambers S.T., et al. Adaptive resistance to tobramycin in Pseudomonas aeruginosa lung infection in cystic fibrosis. J Antimicrob Chemother. 37:1996;1155-1164.
    • (1996) J Antimicrob Chemother , vol.37 , pp. 1155-1164
    • Barclay, M.L.1    Begg, E.J.2    Chambers, S.T.3
  • 92
    • 0035226425 scopus 로고    scopus 로고
    • Elective versus symptomatic intravenous antibiotic therapy for cystic fibrosis
    • Breen L., Aswani N. Elective versus symptomatic intravenous antibiotic therapy for cystic fibrosis. Cochrane Database Syst Rev. 4:2001;CD002767.
    • (2001) Cochrane Database Syst Rev , vol.4 , pp. 002767
    • Breen, L.1    Aswani, N.2
  • 93
    • 0029921514 scopus 로고    scopus 로고
    • Improved survival in the Danish center-treated cystic fibrosis patients: Results of aggressive treatment
    • Frederiksen B., Lanng S., Koch C., et al. Improved survival in the Danish center-treated cystic fibrosis patients: results of aggressive treatment. Pediatr Pulmonol. 21:1996;153.
    • (1996) Pediatr Pulmonol , vol.21 , pp. 153
    • Frederiksen, B.1    Lanng, S.2    Koch, C.3
  • 94
    • 0034107646 scopus 로고    scopus 로고
    • Elective versus symptomatic antibiotic treatment in cystic fibrosis patients with chronic Pseudomonas infection of the lungs
    • Elborn J.S., Prescott R.J., Stack B.H., et al. Elective versus symptomatic antibiotic treatment in cystic fibrosis patients with chronic Pseudomonas infection of the lungs. Thorax. 55:2000;355-358.
    • (2000) Thorax , vol.55 , pp. 355-358
    • Elborn, J.S.1    Prescott, R.J.2    Stack, B.H.3
  • 95
    • 0024488435 scopus 로고
    • Long-term tobramycin inhalation in cystic fibrosis
    • Steinkamp G., Tümmler H., Gappa M., et al. Long-term tobramycin inhalation in cystic fibrosis. Pediatr Pulmonol. 6:1989;91-98.
    • (1989) Pediatr Pulmonol , vol.6 , pp. 91-98
    • Steinkamp, G.1    Tümmler, H.2    Gappa, M.3
  • 96
    • 0023265976 scopus 로고
    • Colistin inhalation therapy in cystic fibrosis patients with chronic P aeruginosa infection
    • Jensen T., Pedersen S.S., Garne S., et al. Colistin inhalation therapy in cystic fibrosis patients with chronic P aeruginosa infection. J Antimicrob Chemother. 19:1987;831-838.
    • (1987) J Antimicrob Chemother , vol.19 , pp. 831-838
    • Jensen, T.1    Pedersen, S.S.2    Garne, S.3
  • 97
    • 0033531143 scopus 로고    scopus 로고
    • Intermittent administration of inhaled tobramycin in patients with cystic fibrosis
    • Ramsey B.W., Pepe M.S., Quan J.M., et al. Intermittent administration of inhaled tobramycin in patients with cystic fibrosis. N Engl J Med. 340:1999;23-30.
    • (1999) N Engl J Med , vol.340 , pp. 23-30
    • Ramsey, B.W.1    Pepe, M.S.2    Quan, J.M.3
  • 98
    • 84921431098 scopus 로고    scopus 로고
    • Nebulised anti-pseudomonal antibiotics for cystic fibrosis (Cochrane Review)
    • Ryan G., Mukhopadhyay S., Singh M. Nebulised anti-pseudomonal antibiotics for cystic fibrosis (Cochrane Review). Cochrane Database Syst Rev. 2:2000;CD001021.
    • (2000) Cochrane Database Syst Rev , vol.2 , pp. 001021
    • Ryan, G.1    Mukhopadhyay, S.2    Singh, M.3
  • 99
    • 0031807730 scopus 로고    scopus 로고
    • Improvement of survival in patients with diffuse panbronchiolitis treated with low-dose erythromycin
    • Kudoh S., Azuma A., Yamamoto M., et al. Improvement of survival in patients with diffuse panbronchiolitis treated with low-dose erythromycin. Am J Respir Crit Care Med. 157:1998;1829-1832.
    • (1998) Am J Respir Crit Care Med , vol.157 , pp. 1829-1832
    • Kudoh, S.1    Azuma, A.2    Yamamoto, M.3
  • 100
    • 0344624839 scopus 로고    scopus 로고
    • Long-term azithromycin may improve lung function in children with cystic fibrosis
    • Jaffe A., Francis J., Rosenthal M., et al. Long-term azithromycin may improve lung function in children with cystic fibrosis. Lancet. 351:1998;420.
    • (1998) Lancet , vol.351 , pp. 420
    • Jaffe, A.1    Francis, J.2    Rosenthal, M.3
  • 101
    • 0036185807 scopus 로고    scopus 로고
    • Effect of long term treatment with azithromycin on disease parameters in cystic fibrosis: A randomised trial
    • Wolter J., Seeney S., Bell S., et al. Effect of long term treatment with azithromycin on disease parameters in cystic fibrosis: a randomised trial. Thorax. 57:2002;212-216.
    • (2002) Thorax , vol.57 , pp. 212-216
    • Wolter, J.1    Seeney, S.2    Bell, S.3
  • 102
    • 0037190575 scopus 로고    scopus 로고
    • Long term azithromycin in children with cystic fibrosis: A randomised, placebo-controlled crossover trial
    • Equi A., Balfour-Lynn I., Bush A., Rosenthal M. Long term azithromycin in children with cystic fibrosis: a randomised, placebo-controlled crossover trial. Lancet. 360:2002;978-984.
    • (2002) Lancet , vol.360 , pp. 978-984
    • Equi, A.1    Balfour-Lynn, I.2    Bush, A.3    Rosenthal, M.4
  • 103
  • 104
    • 0021996644 scopus 로고
    • Nebulised colomycin for early Pseudomonas colonisation in cystic fibrosis
    • Littlewood J.M., Miller M.G., Ghoneim A.T., et al. Nebulised colomycin for early Pseudomonas colonisation in cystic fibrosis. Lancet. 1:1985;865.
    • (1985) Lancet , vol.1 , pp. 865
    • Littlewood, J.M.1    Miller, M.G.2    Ghoneim, A.T.3
  • 105
    • 0025868478 scopus 로고
    • Prevention of chronic Pseudomonas aeruginosa colonisation by early treatment
    • Valerius N.H., Koch C., Hoiby N. Prevention of chronic Pseudomonas aeruginosa colonisation by early treatment. Lancet. 338:1991;725-726.
    • (1991) Lancet , vol.338 , pp. 725-726
    • Valerius, N.H.1    Koch, C.2    Hoiby, N.3
  • 106
    • 2642712510 scopus 로고    scopus 로고
    • Placebo-controlled double-blind randomized study of aerosolized tobramycin for early treatment of Pseudomonas aeruginosa colonisation in cystic fibrosis
    • Wiesemann H.G., Steinkamp G., Ratjen F., et al. Placebo-controlled double-blind randomized study of aerosolized tobramycin for early treatment of Pseudomonas aeruginosa colonisation in cystic fibrosis. Pediatr Pulmonol. 26:1998;88-92.
    • (1998) Pediatr Pulmonol , vol.26 , pp. 88-92
    • Wiesemann, H.G.1    Steinkamp, G.2    Ratjen, F.3
  • 107
    • 0035934545 scopus 로고    scopus 로고
    • Eradication of Pseudomonas aeruginosa with inhaled tobramycin in patients with cystic fibrosis
    • Ratjen F., Döring G., Nikolaizik W. Eradication of Pseudomonas aeruginosa with inhaled tobramycin in patients with cystic fibrosis. Lancet. 358:2001;983-984.
    • (2001) Lancet , vol.358 , pp. 983-984
    • Ratjen, F.1    Döring, G.2    Nikolaizik, W.3
  • 108
    • 0021970474 scopus 로고
    • Alternate-day prednisone reduces morbitity and improves pulmonary function in cystic fibrosis
    • Auerbach H.S., Willaims M., Kirkpatrick J.A., et al. Alternate-day prednisone reduces morbitity and improves pulmonary function in cystic fibrosis. Lancet. 2:1985;686-688.
    • (1985) Lancet , vol.2 , pp. 686-688
    • Auerbach, H.S.1    Willaims, M.2    Kirkpatrick, J.A.3
  • 109
    • 0028969594 scopus 로고
    • A multicenter study of alternate-day prednisone therapy in patients with cystic fibrosis
    • Eigen H., Rosenstein B.J., Fitzsimmons S., et al. A multicenter study of alternate-day prednisone therapy in patients with cystic fibrosis. J Pediatr. 126:1995;515-523.
    • (1995) J Pediatr , vol.126 , pp. 515-523
    • Eigen, H.1    Rosenstein, B.J.2    Fitzsimmons, S.3
  • 110
    • 0034704799 scopus 로고    scopus 로고
    • FitzSimmons SC, Allen DB, et al. Risk of persistent growth impairment after alternate-day prednisone treatment in children with cystic fibrosis
    • Lai H.C. FitzSimmons SC, Allen DB, et al. Risk of persistent growth impairment after alternate-day prednisone treatment in children with cystic fibrosis. N Engl J Med. 342:2000;851-859.
    • (2000) N Engl J Med , vol.342 , pp. 851-859
    • Lai, H.C.1
  • 111
    • 84921431158 scopus 로고    scopus 로고
    • Oral steroids for cystic fibrosis (Cochrane Review)
    • Cheng K., Ashby D., Smyth R. Oral steroids for cystic fibrosis (Cochrane Review). Cochrane Database Syst Rev. 2:2000;CD000407.
    • (2000) Cochrane Database Syst Rev , vol.2 , pp. 000407
    • Cheng, K.1    Ashby, D.2    Smyth, R.3
  • 113
    • 0028914667 scopus 로고
    • Effect of high-dose ibuprofen in patients with cystic fibrosis
    • Konstan M.W., Byard P.J., Hoppel C.L., et al. Effect of high-dose ibuprofen in patients with cystic fibrosis. N Engl J Med. 332:1995;848-854.
    • (1995) N Engl J Med , vol.332 , pp. 848-854
    • Konstan, M.W.1    Byard, P.J.2    Hoppel, C.L.3
  • 114
    • 0000879923 scopus 로고    scopus 로고
    • Immunology of cystic fibrosis
    • M.E. Hodson, & D. Geddes. London: Arnold
    • Döring G., Knight R., Bellon G. Immunology of cystic fibrosis. Hodson M.E., Geddes D. Cystic fibrosis. 2000;109-141 Arnold, London.
    • (2000) Cystic fibrosis , pp. 109-141
    • Döring, G.1    Knight, R.2    Bellon, G.3
  • 115
    • 0026474443 scopus 로고
    • Modulation of airway inflammation in cystic fibrosis: In vivo suppression of interleukin-8 levels on the respiratory epithelial surface by aerosolization of recombinant secretory leukoprotease inhibitor
    • McElvaney N.G., Nakamura H., Birrer P., et al. Modulation of airway inflammation in cystic fibrosis: in vivo suppression of interleukin-8 levels on the respiratory epithelial surface by aerosolization of recombinant secretory leukoprotease inhibitor. J Clin Invest. 90:1992;1296-1301.
    • (1992) J Clin Invest , vol.90 , pp. 1296-1301
    • McElvaney, N.G.1    Nakamura, H.2    Birrer, P.3
  • 116
    • 0027495712 scopus 로고
    • Pharmacokinetics of recombinant secretory leukoprotease inhibitor aerosolized to normals and individuals with cystic fibrosis
    • McElvaney N.G., Doujaiji B., Moan M.J., et al. Pharmacokinetics of recombinant secretory leukoprotease inhibitor aerosolized to normals and individuals with cystic fibrosis. Am Rev Respir Dis. 148:1993;1056-1060.
    • (1993) Am Rev Respir Dis , vol.148 , pp. 1056-1060
    • McElvaney, N.G.1    Doujaiji, B.2    Moan, M.J.3
  • 118
    • 0032882814 scopus 로고    scopus 로고
    • Aerosolized prolastin suppresses bacterial proliferation in a model of chronic Pseudomonas aeruginosa lung infection
    • Cantin A.M., Woods D.E. Aerosolized prolastin suppresses bacterial proliferation in a model of chronic Pseudomonas aeruginosa lung infection. Am J Respir Crit Care Med. 160:1999;1130-1135.
    • (1999) Am J Respir Crit Care Med , vol.160 , pp. 1130-1135
    • Cantin, A.M.1    Woods, D.E.2
  • 119
    • 0022378715 scopus 로고
    • A double-blind placebo controlled trial with oral ambroxol and n-acetylcysteine for mucolytic treatment in cystic fibrosis
    • Ratjen F., Wönne R., Posselt H.G., et al. A double-blind placebo controlled trial with oral ambroxol and n-acetylcysteine for mucolytic treatment in cystic fibrosis. Eur J Pediatr. 144:1985;374-378.
    • (1985) Eur J Pediatr , vol.144 , pp. 374-378
    • Ratjen, F.1    Wönne, R.2    Posselt, H.G.3
  • 120
    • 0028129568 scopus 로고
    • Effect of aerosolized recombinant human DNase on exacerbations of respiratory symptoms and on pulmonary function in patients with cystic fibrosis
    • Fuchs H.J., Borowitz D.S., Christiansen D.H., et al. Effect of aerosolized recombinant human DNase on exacerbations of respiratory symptoms and on pulmonary function in patients with cystic fibrosis. N Engl J Med. 331:1994;637-642.
    • (1994) N Engl J Med , vol.331 , pp. 637-642
    • Fuchs, H.J.1    Borowitz, D.S.2    Christiansen, D.H.3
  • 121
    • 0035666058 scopus 로고    scopus 로고
    • A two-year randomized, placebo-controlled trial of dornase alfa in young patients with cystic fibrosis with mild lung function abnormalities
    • Quan J.M., Tiddens H.A., Sy J.P., et al. A two-year randomized, placebo-controlled trial of dornase alfa in young patients with cystic fibrosis with mild lung function abnormalities. J Pediatr. 139:2001;813-820.
    • (2001) J Pediatr , vol.139 , pp. 813-820
    • Quan, J.M.1    Tiddens, H.A.2    Sy, J.P.3
  • 122
    • 84921431060 scopus 로고    scopus 로고
    • Deoxyribonuclease for cystic fibrosis (Cochrane Review)
    • Kearney C.E., Wallis C.E. Deoxyribonuclease for cystic fibrosis (Cochrane Review). Cochrane Database Syst Rev. 2:2000;CD001127.
    • (2000) Cochrane Database Syst Rev , vol.2 , pp. 001127
    • Kearney, C.E.1    Wallis, C.E.2
  • 123
    • 0035922668 scopus 로고    scopus 로고
    • Comparison of hypertonic saline and alternate-day or daily recombinant human deoxyribonuclease in children with cystic fibrosis: A randomised trial
    • Suri R., Metcalfe C., Lees B., et al. Comparison of hypertonic saline and alternate-day or daily recombinant human deoxyribonuclease in children with cystic fibrosis: a randomised trial. Lancet. 358:2001;1316-1321.
    • (2001) Lancet , vol.358 , pp. 1316-1321
    • Suri, R.1    Metcalfe, C.2    Lees, B.3
  • 124
    • 84921430394 scopus 로고    scopus 로고
    • Nebulised hypertonic saline for cystic fibrosis (Cochrane Review)
    • Wark P.A., McDonald V. Nebulised hypertonic saline for cystic fibrosis (Cochrane Review). Cochrane Database Syst Rev. 2:2000;CD001506.
    • (2000) Cochrane Database Syst Rev , vol.2 , pp. 001506
    • Wark, P.A.1    McDonald, V.2
  • 125
    • 0034570842 scopus 로고    scopus 로고
    • Worldwide thoracic organ transplantation: A report from the UNOS/ISHLT international Registry for Thoracic Organ Transplantation
    • Bennet L.E., Keck B.M., Daily O.P., et al. Worldwide thoracic organ transplantation: a report from the UNOS/ISHLT international Registry for Thoracic Organ Transplantation. Clin Transpl. 1:2000;31-44.
    • (2000) Clin Transpl , vol.1 , pp. 31-44
    • Bennet, L.E.1    Keck, B.M.2    Daily, O.P.3
  • 126
    • 0035814353 scopus 로고    scopus 로고
    • Survival effect of lung transplantation among patients with cystic fibrosis
    • Liou T.G., Adler F.R., Cahill B.C., et al. Survival effect of lung transplantation among patients with cystic fibrosis. JAMA. 286:2001;2683-2689.
    • (2001) JAMA , vol.286 , pp. 2683-2689
    • Liou, T.G.1    Adler, F.R.2    Cahill, B.C.3
  • 127
    • 0033530379 scopus 로고    scopus 로고
    • Lung transplantation and life extension in children with cystic fibrosis
    • Aurora P., Whitehead B., Wade A., et al. Lung transplantation and life extension in children with cystic fibrosis. Lancet. 354:1999;1591-1593.
    • (1999) Lancet , vol.354 , pp. 1591-1593
    • Aurora, P.1    Whitehead, B.2    Wade, A.3
  • 128
    • 0035659459 scopus 로고    scopus 로고
    • Lung transplantation for cystic fibrosis patients with Burkholderia cepacia complex: Survival linked to genomovar type
    • Aris R.M., Routh J.C., LiPuma J.J., et al. Lung transplantation for cystic fibrosis patients with Burkholderia cepacia complex: survival linked to genomovar type. Am J Respir Crit Care Med. 164:2001;2102-2106.
    • (2001) Am J Respir Crit Care Med , vol.164 , pp. 2102-2106
    • Aris, R.M.1    Routh, J.C.2    LiPuma, J.J.3
  • 129
    • 0026520359 scopus 로고
    • Prediction of mortality in patients with cystic fibrosis
    • Kerem E., Reisman J., Corey M., et al. Prediction of mortality in patients with cystic fibrosis. N Engl J Med. 326:1992;1187-1191.
    • (1992) N Engl J Med , vol.326 , pp. 1187-1191
    • Kerem, E.1    Reisman, J.2    Corey, M.3
  • 130
    • 0035053808 scopus 로고    scopus 로고
    • Living donor lung transplantation
    • Cohen R.G., Starnes V.A. Living donor lung transplantation. World J Surg. 25:2001;244-250.
    • (2001) World J Surg , vol.25 , pp. 244-250
    • Cohen, R.G.1    Starnes, V.A.2
  • 131
    • 0020070795 scopus 로고
    • Improved respiratory prognosis in patients with cystic fibrosis with normal fat absorption
    • Gaskin K., Gurwitz D., Durie P., et al. Improved respiratory prognosis in patients with cystic fibrosis with normal fat absorption. J Pediatr. 100:1982;857-862.
    • (1982) J Pediatr , vol.100 , pp. 857-862
    • Gaskin, K.1    Gurwitz, D.2    Durie, P.3
  • 132
    • 0023923679 scopus 로고
    • A comparison of survival, growth, and pulmonary function in patients with cystic fibrosis in Boston and Toronto
    • Corey M., McLaughlin F.J., Williams M., et al. A comparison of survival, growth, and pulmonary function in patients with cystic fibrosis in Boston and Toronto. J Clin Epidemiol. 41:1988;583-591.
    • (1988) J Clin Epidemiol , vol.41 , pp. 583-591
    • Corey, M.1    McLaughlin, F.J.2    Williams, M.3
  • 133
    • 0036299705 scopus 로고    scopus 로고
    • Nutrition in patients with cystic fibrosis: A European consensus
    • Sinaasappel M., Stern M., Littlewood J., et al. Nutrition in patients with cystic fibrosis: a European consensus. J Cystic Fibrosis. 1:2002;51-75.
    • (2002) J Cystic Fibrosis , vol.1 , pp. 51-75
    • Sinaasappel, M.1    Stern, M.2    Littlewood, J.3
  • 134
    • 84921430676 scopus 로고    scopus 로고
    • Oral calorie supplements for cystic fibrosis (Cochrane Review)
    • Smyth R., Walters S. Oral calorie supplements for cystic fibrosis (Cochrane Review). Cochrane Database Syst Rev. 2:2000;CD000406.
    • (2000) Cochrane Database Syst Rev , vol.2 , pp. 000406
    • Smyth, R.1    Walters, S.2
  • 135
    • 44949089717 scopus 로고    scopus 로고
    • Enteral tube feeding for cystic fibrosis (Cochrane Review)
    • Conway S.P., Morton A., Wolfe S. Enteral tube feeding for cystic fibrosis (Cochrane Review). Cochrane Database Syst Rev. 2:2000;CD001198.
    • (2000) Cochrane Database Syst Rev , vol.2 , pp. 001198
    • Conway, S.P.1    Morton, A.2    Wolfe, S.3
  • 136
    • 84921431266 scopus 로고    scopus 로고
    • Ursodeoxycholic acid for cystic fibrosis-related liver disease (Cochrane Review)
    • Cheng K., Ashby D., Smyth R. Ursodeoxycholic acid for cystic fibrosis-related liver disease (Cochrane Review). Cochrane Database Syst Rev. 2:2000;CD000222.
    • (2000) Cochrane Database Syst Rev , vol.2 , pp. 000222
    • Cheng, K.1    Ashby, D.2    Smyth, R.3
  • 137
    • 0036230252 scopus 로고    scopus 로고
    • Transplantation for cystic fibrosis: Outcome following early liver transplantation
    • Milkiewicz P., Skiba G., Kelly D., et al. Transplantation for cystic fibrosis: outcome following early liver transplantation. J Gastroenterol Hepatol. 17:2002;208-213.
    • (2002) J Gastroenterol Hepatol , vol.17 , pp. 208-213
    • Milkiewicz, P.1    Skiba, G.2    Kelly, D.3
  • 138
    • 0034046150 scopus 로고    scopus 로고
    • International comparison of median age at death from cystic fibrosis
    • Fogarthy A., Hubbard R., Britton J. International comparison of median age at death from cystic fibrosis. Chest. 117:2000;1656-1660.
    • (2000) Chest , vol.117 , pp. 1656-1660
    • Fogarthy, A.1    Hubbard, R.2    Britton, J.3
  • 140
    • 0034987271 scopus 로고    scopus 로고
    • The association of socioeconomic status with outcomes in cystic fibrosis patients in the United States
    • Schechter M.S., Shelton B.J., Margolis P.A., et al. The association of socioeconomic status with outcomes in cystic fibrosis patients in the United States. Am J Respir Crit Care Med. 163:2001;1331-1337.
    • (2001) Am J Respir Crit Care Med , vol.163 , pp. 1331-1337
    • Schechter, M.S.1    Shelton, B.J.2    Margolis, P.A.3


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